Structural Progression in Patients with Definite and Non-Definite Arrhythmogenic Right Ventricular Cardiomyopathy and Risk of Major Adverse Cardiac Events

Areej Aljehani; Shanat Baig; Tania Kew; Manish Kalla; Laura C. Sommerfeld; Vaishnavi Ameya Murukutla; Larissa Fabritz; Richard P. Steeds

doi:10.3390/biomedicines12020328

Structural Progression in Patients with Definite and Non-Definite Arrhythmogenic Right Ventricular Cardiomyopathy and Risk of Major Adverse Cardiac Events

Areej Aljehani, Shanat Baig, Tania Kew, Manish Kalla, Laura C. Sommerfeld, Vaishnavi Ameya Murukutla, Larissa Fabritz^*, Richard P. Steeds

^*Corresponding author for this work

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Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease characterised by early arrhythmias and structural changes. Still, there are limited echocardiography data on its structural progression. We studied structural progression and its impact on the occurrence of major adverse cardiovascular events (MACE). In this single-centre observational cohort study, structural progression was defined as the development of new major or minor imaging 2010 Task Force Criteria during follow-up. Of 101 patients, a definite diagnosis of ARVC was made in 51 patients, while non-definite ‘early’ disease was diagnosed in 50 patients. During 4 years of follow-up (IQR: 2–6), 23 (45%) patients with a definite diagnosis developed structural progression while only 1 patient in the non-definite (early) group gained minor imaging Task Force Criteria. Male gender was strongly associated with structural progression (62% of males progressed structurally, while 88% of females remained stable). Patients with structural progression were at higher risk of MACE (64% of patients with MACE had structural progression). Therefore, the rate of structural progression is an essential factor to be considered in ARVC studies.

Original language	English
Article number	328
Journal	Biomedicines
Volume	12
Issue number	2
DOIs	https://doi.org/10.3390/biomedicines12020328
Publication status	Published - 31 Jan 2024

Bibliographical note

EU Horizon 2020 MAESTRIA (grant agreement number 965286 to LF), The National Institute of Health and Care Research (NIHR) Birmingham Biomedical Research Centre (NIHR203326) and the British Heart Foundation Accelerator (BHF), (AA/18/2/34218) have supported the University of Birmingham Institute of Cardiovascular Sciences where this research is based. The opinions expressed in this paper are those of the authors and do not represent any of the listed organisations’. AA’s PhD stipend is funded by the Royal Embassy of Saudi Arabia Cultural Bureau. RS acknowledges partial funding from the NIHR West Midlands Senior Clinical Scholarship, LF from DZHK.

Keywords

echocardiography
male sex
major adverse cardiac events
structural progression
disease progression
sudden cardiac death
arrhythmogenic right ventricular cardiomyopathy
single-centre observational cohort study

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Cite this

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title = "Structural Progression in Patients with Definite and Non-Definite Arrhythmogenic Right Ventricular Cardiomyopathy and Risk of Major Adverse Cardiac Events",

abstract = "Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease characterised by early arrhythmias and structural changes. Still, there are limited echocardiography data on its structural progression. We studied structural progression and its impact on the occurrence of major adverse cardiovascular events (MACE). In this single-centre observational cohort study, structural progression was defined as the development of new major or minor imaging 2010 Task Force Criteria during follow-up. Of 101 patients, a definite diagnosis of ARVC was made in 51 patients, while non-definite {\textquoteleft}early{\textquoteright} disease was diagnosed in 50 patients. During 4 years of follow-up (IQR: 2–6), 23 (45%) patients with a definite diagnosis developed structural progression while only 1 patient in the non-definite (early) group gained minor imaging Task Force Criteria. Male gender was strongly associated with structural progression (62% of males progressed structurally, while 88% of females remained stable). Patients with structural progression were at higher risk of MACE (64% of patients with MACE had structural progression). Therefore, the rate of structural progression is an essential factor to be considered in ARVC studies.",

keywords = "echocardiography, male sex, major adverse cardiac events, structural progression, disease progression, sudden cardiac death, arrhythmogenic right ventricular cardiomyopathy, single-centre observational cohort study",

author = "Areej Aljehani and Shanat Baig and Tania Kew and Manish Kalla and Sommerfeld, {Laura C.} and Murukutla, {Vaishnavi Ameya} and Larissa Fabritz and Steeds, {Richard P.}",

note = "EU Horizon 2020 MAESTRIA (grant agreement number 965286 to LF), The National Institute of Health and Care Research (NIHR) Birmingham Biomedical Research Centre (NIHR203326) and the British Heart Foundation Accelerator (BHF), (AA/18/2/34218) have supported the University of Birmingham Institute of Cardiovascular Sciences where this research is based. The opinions expressed in this paper are those of the authors and do not represent any of the listed organisations{\textquoteright}. AA{\textquoteright}s PhD stipend is funded by the Royal Embassy of Saudi Arabia Cultural Bureau. RS acknowledges partial funding from the NIHR West Midlands Senior Clinical Scholarship, LF from DZHK.",

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AU - Aljehani, Areej

AU - Baig, Shanat

AU - Kew, Tania

AU - Kalla, Manish

AU - Sommerfeld, Laura C.

AU - Murukutla, Vaishnavi Ameya

AU - Fabritz, Larissa

AU - Steeds, Richard P.

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N2 - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease characterised by early arrhythmias and structural changes. Still, there are limited echocardiography data on its structural progression. We studied structural progression and its impact on the occurrence of major adverse cardiovascular events (MACE). In this single-centre observational cohort study, structural progression was defined as the development of new major or minor imaging 2010 Task Force Criteria during follow-up. Of 101 patients, a definite diagnosis of ARVC was made in 51 patients, while non-definite ‘early’ disease was diagnosed in 50 patients. During 4 years of follow-up (IQR: 2–6), 23 (45%) patients with a definite diagnosis developed structural progression while only 1 patient in the non-definite (early) group gained minor imaging Task Force Criteria. Male gender was strongly associated with structural progression (62% of males progressed structurally, while 88% of females remained stable). Patients with structural progression were at higher risk of MACE (64% of patients with MACE had structural progression). Therefore, the rate of structural progression is an essential factor to be considered in ARVC studies.

AB - Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease characterised by early arrhythmias and structural changes. Still, there are limited echocardiography data on its structural progression. We studied structural progression and its impact on the occurrence of major adverse cardiovascular events (MACE). In this single-centre observational cohort study, structural progression was defined as the development of new major or minor imaging 2010 Task Force Criteria during follow-up. Of 101 patients, a definite diagnosis of ARVC was made in 51 patients, while non-definite ‘early’ disease was diagnosed in 50 patients. During 4 years of follow-up (IQR: 2–6), 23 (45%) patients with a definite diagnosis developed structural progression while only 1 patient in the non-definite (early) group gained minor imaging Task Force Criteria. Male gender was strongly associated with structural progression (62% of males progressed structurally, while 88% of females remained stable). Patients with structural progression were at higher risk of MACE (64% of patients with MACE had structural progression). Therefore, the rate of structural progression is an essential factor to be considered in ARVC studies.

KW - echocardiography

KW - male sex

KW - major adverse cardiac events

KW - structural progression

KW - disease progression

KW - sudden cardiac death

KW - arrhythmogenic right ventricular cardiomyopathy

KW - single-centre observational cohort study

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DO - 10.3390/biomedicines12020328

M3 - Article

SN - 2227-9059

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JO - Biomedicines

JF - Biomedicines

IS - 2

M1 - 328

ER -

Structural Progression in Patients with Definite and Non-Definite Arrhythmogenic Right Ventricular Cardiomyopathy and Risk of Major Adverse Cardiac Events

Abstract

Bibliographical note

Keywords

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